Haem 12: Myeloma etc Flashcards

1
Q

What is myelomas?

A

plasma cell dyscrasia - cancer caused by abnormal proliferation of plasma cells

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2
Q

What happens in myeloma?

A

Clonal population of plasma cells which proliferate and produce monoclonal immunoglobulin light chains:

In blood, found as paraprotein
In urine, found as Bence Jones Protein

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3
Q

What is the relationship between MGUS, smoldering myeloma and multiple myeloma?

A

MGUS and smoldering myeloma can both progress to multiple myeloma

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4
Q

What is the definition of multiple myeloma + the symptoms of multiple myeloma?

A

Clonal bone marrow plasma cells >10% in the marrow or plasmacytoma
And end organ damage-> CRAB;

Calcium: Hypercalcaemia >2.75 
Renal: Creatinine clearance <40ml/min / Creatinine >177
Anaemia Hb <100g/l
Bone lesions – lytic lesions
(+ monoclonal protein)
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5
Q

What is the definition + features of smouldering myeloma? Prognosis?

A

Serum monoclonal protein (IgG / IgA) >30g/l (for paraprotein as well) or Bence Jones Protein

And/or Clonal bone marrow plasma cells 10%-60% in the marrow

No end-organ damage (NO CRAB)

Most will progress to myeloma untreated

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6
Q

What is MGUS + Prognosis?

A

Monoclonal Gammopathy of Unknown Significance (MGUS)

Serum monoclonal protein <30g/l
Plasma cells <10% on Bone Marrow

No end organ damage
No evidence of other disorder

1-2% per year progress to myeloma

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7
Q

Differences between MGUS, Smouldering Myeloma, and Multiple Myeloma (paraprotein, plasma in BM, CRAM, Leisons on MRI, free light chain ratio, progression rate)

A
MGUS:
Paraprotein <30
Plasma cells in BM <10%
No CRAB or leisons on MRI
Free lgith chain ratio <100
Progression = 1-2% to MM
Smouldering Myeloma:
Paraprotein >30
Plasma cells in BM 10-60%
No CRAB or leisons on MRI
Free light chain ratio <100
Progression = 10% to MM
MM:
Paraprotein >30 OR
Plasma cells in BM >10% OR
CRAB or leisons on MRI PRESENT
Free light chain ratio >100
Progression = 10% to MM
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8
Q

What can you see in blood films in myeloma?

A

Rouleaux formation - stacked RBCs

plasma cells in blood film is only present in v late disease

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9
Q

Mx of MGUS?

A

Anual blood test

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10
Q

Mx of Smouldering Myeloma?

A

May not always but in most cases you would treat

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11
Q

Mx of MM?

A

Treat

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12
Q

What does myeloma ix involve?

A

Investigation of paraprotein (if low level may not do all)
Urine bence jones
Whole body imaging to rule out lytic lesions
Calcium, Renal function, Full blood count
Bone marrow

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13
Q

General principles of myeloma mx?

A

Younger patients: chemotherapy then autologous stem cell transplant

Older patients: chemotherapy then maintenance therapy

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14
Q

What are autologous hematopoietic stem cell transplants?

A

Collect patient’s own stem cells

Give them high dose chemo to destroy bone marrow (and hopefully any malignancy left)

Re-infuse stem cells

more commonly used in myelomas

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15
Q

What are allogenic hematopoietic stem cell transplants?

A

Collect relative / unrelated donor cells

Give patient high dose chemotherapy to destroy remaining cancer AND patient’s immune cells to avoid rejection

Infuse stem cells

More commonly used in leukaemias

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16
Q

What is Waldenstrom’s Macroglobulinaemia?

A

This is a condition related to MM - can present w/ CRAB + paraprotein

However in these pts the paraprotein is IgM!!! esp if w/ lymphadenopathy (more like a lymphoma)